11 research outputs found
Cystic Fibrosis Patients Eligible for Modulator Drugs: Data from Cystic Fibrosis Registry of Turkey
TÜRKIYE’DE SON BIR YILDA İZLENEN KISTIKFIBROZIS HASTALARI: ULUSAL KISTIK FIBROZISKAYIT SISTEMI’NDEN İLK VERILER
Clinical Features of Cystic Fibrosis Patients with Chronic Liver Disease in the Turkish National Cystic Fibrosis Registry
Evaluation of factors affecting bone mineral density in CF patients through CF registry of Turkey
ULUSAL KISTIK FIBROZIS KAYIT SISTEMINDE KAYITLI KRONIK KARACIĞER HASTALIĞI OLAN KISTIK FIBROZISLI HASTALARIN KLINIK ÖZELLIKLERI
Childhood interstitial lung disease in Turkey: first data from the national registry
Abstract: The childhood interstitial lung diseases (chILD) Turkey registry (chILD-TR) was established in November 2021 to increase awareness of disease, and in collaboration with the centers to improve the diagnostic and treatment standards. Here, the first results of the chILD registry system were presented. In this prospective cohort study, data were collected using a data‐entry software system. The demographic characteristics, clinical, laboratory, radiologic findings, diagnoses, and treatment characteristics of the patients were evaluated. Clinical characteristics were compared between two main chILD groups ((A) diffuse parenchymal lung diseases (DPLD) disorders manifesting primarily in infancy [group1] and (B) DPLD disorders occurring at all ages [group 2]). There were 416 patients registered from 19 centers. Forty-six patients were excluded due to missing information. The median age of diagnosis of the patients was 6.05 (1.3–11.6) years. Across the study population (n = 370), 81 (21.8%) were in group 1, and 289 (78.1%) were in group 2. The median weight z-score was significantly lower in group 1 (− 2.0 [− 3.36 to − 0.81]) than in group 2 (− 0.80 [− 1.7 to 0.20]) (p < 0.001). When we compared the groups according to chest CT findings, ground-glass opacities were significantly more common in group 1, and nodular opacities, bronchiectasis, mosaic perfusion, and mediastinal lymphadenopathy were significantly more common in group 2. Out of the overall study population, 67.8% were undergoing some form of treatment. The use of oral steroids was significantly higher in group 2 than in group 1 (40.6% vs. 23.3%, respectively; p = 0.040). Conclusion: This study showed that national registry allowed to obtain information about the frequency, types, and treatment methods of chILD in Turkey and helped to see the difficulties in the diagnosis and management of these patients. What is Known: • Childhood interstitial lung diseases comprise many diverse entities which are challenging to diagnose and manage. What is New: • This study showed that national registry allowed to obtain information about the frequency, types and treatment methods of chILD in Turkey and helped to see the difficulties in the diagnosis and management of these patients. Also, our findings reveal that nutrition should be considered in all patients with chILD, especially in A-DPLD disorders manifesting primarily in infancy